Imatinib Mesylate in the Treatment of Refractory Idiopathic Pulmonary Arterial Hypertension

Letters18 July 2006Imatinib Mesylate in the Treatment of Refractory Idiopathic Pulmonary Arterial HypertensionKaren C. Patterson, MD, Ariel Weissmann, MD, MPH, Tahamtan Ahmadi, MD, PhD, and Harrison W. Farber, MDKaren C. Patterson, MDFrom Boston Medical Center, Boston, MA 02118., Ariel Weissmann, MD, MPHFrom Boston Medical Center, Boston, MA 02118., Tahamtan Ahmadi, MD, PhDFrom Boston Medical Center, Boston, MA 02118., and Harrison W. Farber, MDFrom Boston Medical Center, Boston, MA 02118.Author, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-145-2-200607180-00020 SectionsAboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail Background: Idiopathic pulmonary arterial hypertension (PAH) is a pathologic process of inappropriate vasoconstriction, vascular remodeling with smooth muscle cell hypertrophy, and in situ thrombosis, which eventually leads to right-heart failure and death. Current treatments primarily attenuate vasoconstriction and do not substantially reduce morbidity and mortality rates.Objective: To describe a patient with severe, refractory idiopathic PAH that did not respond to conventional treatment but did show clinical and hemodynamic response to imatinib mesylate therapy, which may affect vascular remodeling.Case Report: A 52-year-old man with rapidly progressive idiopathic PAH was evaluated for progressive dyspnea on exertion. Echocardiography and right-heart catheterization ...References1. Tapson VF, Gomberg-Maitland M, McLaughlin VV, Benza RL, Widlitz AC, Krichman A, et al. Safety and efficacy of IV treprostinil for pulmonary arterial hypertension: a prospective, multicenter, open-label, 12-week trial. Chest. 2006;129:683-8. [PMID: 16537868] CrossrefMedlineGoogle Scholar2. Galie N, Ghofrani HA, Torbicki A, Barst RJ, Rubin LJ, Badesch D, et al. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med. 2005;353:2148-57. [PMID: 16291984] CrossrefMedlineGoogle Scholar3. Ghofrani HA, Seeger W, Grimminger F. Imatinib for the treatment of pulmonary arterial hypertension. N Engl J Med. 2005;353:1412-3. [PMID: 16192491] CrossrefMedlineGoogle Scholar4. Humbert M, Monti G, Fartoukh M, Magnan A, Brenot F, Rain B, et al. Platelet-derived growth factor expression in primary pulmonary hypertension: comparison of HIV seropositive and HIV seronegative patients. Eur Respir J. 1998;11:554-9. [PMID: 9596101] MedlineGoogle Scholar5. Schermuly RT, Dony E, Ghofrani HA, Pullamsetti S, Savai R, Roth M, et al. Reversal of experimental pulmonary hypertension by PDGF inhibition. J Clin Invest. 2005;115:2811-21. [PMID: 16200212] CrossrefMedlineGoogle Scholar Author, Article, and Disclosure InformationAuthors: Karen C. Patterson, MD; Ariel Weissmann, MD, MPH; Tahamtan Ahmadi, MD, PhD; Harrison W. Farber, MDAffiliations: From Boston Medical Center, Boston, MA 02118.Disclosures: None disclosed. 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